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Biomedical subjects

M L Bernstein

Publications and source records attributed to M L Bernstein.

At least 19 recordsLinked to original sources
PubMed1992

Cinnamon-induced stomatitis venenata, Clinical and characteristic histopathologic features.

Fourteen new cases of cinnamon-induced stomatitis are reported. Ten of these fourteen cases were first detected on the basis of histopathologic changes, which included hyperkeratosis, chronic lichenoid mucositis with plasmacytic infiltration, and marked chronic perivasculitis. Six cases of false-positive histopathologic findings are presented for comparison. It is recommended that when the histopathologic features described are recognized, cinnamon stomatitis should be considered.

Adult↗
PubMed1992

Screening for neuroblastoma in North America. 2-year results from the Quebec Project.

The Quebec Neuroblastoma Screening Project was initiated to assess the clinical and biological aspects of screening infants for the presence of neuroblastoma in North America. All children born in the province of Quebec from May 1, 1989 to April 30, 1994 are eligible for participation. This report provides results from 22 months' accrual of infants who were screened using urine-saturated filter paper for determination of the catecholamine metabolites vanillylmandelic acid (VMA) and homovanillic acid (HVA). More than 157,000 infants have been screened to date at 3 weeks of age, representing 92% of the entire birth population of Quebec. Over 98,000 infants have been screened a second time at 6 months of age, which made up 76% of the Quebec birth cohort. After a two-stage initial screening, 340 (0.13%) infants (182 at 3 weeks and 158 at 6 months) required second laboratory examinations because of elevated levels of urinary VMA, HVA, or both. Twenty infants from the 3-week screening (0.01%) and nine from the 6-month screening (0.01%) were subsequently referred to one of four Quebec pediatric oncology centers for neuroblastoma evaluation. Seven of 20 children from the 3-week screening and two of nine children from the 6-month screening have been identified as having neuroblastoma. During the same period, 14 additional children in the birth cohort were diagnosed clinically with neuroblastoma; eight were diagnosed prior to screening at 3 weeks of age, three children had negative results at 3 weeks of age, two had negative results at 3 weeks and at 6 months of age, and one had never been screened.(ABSTRACT TRUNCATED AT 250 WORDS)

Algorithms↗
PubMed1992

A population-based study of neuroblastoma incidence, survival, and mortality in North America.

PURPOSE: The purpose of this study was twofold: (1) to provide a population-based estimate of neuroblastoma incidence, disease stage and age distribution, and survival and mortality rates in North America; and (2) to compare these figures in the province of Quebec at a time shortly before the institution of province-wide screening with those in a population-based control group, the Greater Delaware Valley (GDV) Pediatric Tumor Registry. MATERIALS AND METHODS: In Quebec, the four major pediatric teaching hospital records were searched for children with a diagnosis of neuroblastoma. Tumor board registry data and information supplied to the Division of Vital Statistics were also reviewed. Birth statistics were obtained from the population registry. The GDV Pediatric Tumor Registry is a population-based registry of pediatric cancer covering all of Delaware and parts of New Jersey, Pennsylvania, and Maryland. Age, stage of disease, and follow-up data were obtained through December 31, 1989, with Evans neuroblastoma staging data used for all comparisons. RESULTS: One hundred thirty children with neuroblastoma were identified in Quebec and 165 in the GDV, in a combined population of 3,178,736 children. The annual incidence of neuroblastoma was 10.95/10(6) under the age of 15 years and 27.75/10(6) between the ages of 0 and 4 years. The annual mortality rate due to neuroblastoma was 4.89/10(6) and 9.10/10(6) for the age groups 0 to 14 and 0 to 4, respectively. The overall 10-year survival rate for the 295 cases of neuroblastoma was 55%. The 10-year survival rates for patients with Evans stage I-IV and IVS disease were 88%, 90%, 63%, 21%, and 81%. There was no significant difference observed in the incidence, mortality, or survival in the two populations. CONCLUSION: These data represent the first large, population-based description of the clinical presentation and outcome of patients with neuroblastoma in North America, with no significant differences noted between Quebec patients and the GDV patients.

Adolescent↗
PubMed1992

Metastatic neuroblastoma presenting as a mandibular mass.

Neuroblastoma is the third most common type of cancer seen in children, after leukemia and tumours of the central nervous system. Although bony metastasis to the skull and the orbits has been well described, metastasis to the mandible is exceptional; 32 cases have been reported. Two more are presented here, along with a short review of the topic emphasizing the radiographic features and the differential diagnosis.

Abdominal Neoplasms↗
PubMed1990

Phase I trial of indicine-N-oxide in children with leukemia and solid tumors: a Pediatric Oncology Group study.

A phase I trial of indicine-N-oxide was carried out in 12 children with solid tumors and in 16 with leukemia. Doses of 5, 6, and 7.5 g/m2 were given parenterally as a 15-min infusion every 3 weeks. The maximum tolerated dose in patients with solid tumors was 7.5 g/m2 and the dose-limiting toxicity was myelosuppression. In leukemia, the maximum tolerated dose was 6.0 g/m2 and hepatotoxicity was dose-limiting. Half of the children with leukemia showed elevations in transaminase levels and one child died of massive hepatic necrosis. This hepatotoxicity limits the use of indicine-N-oxide in children with leukemia. Antineoplastic activity was limited to a transient reduction in the numbers of circulating leukemic cells.

Adolescent↗
PubMed1990

Hodgkin's disease in the nasopharynx.

Nasal obstruction due to adenoid hypertrophy is extremely common in the pediatric age group and is generally successfully treated by adenoidectomy. The case report of the incidental finding of Hodgkin's disease in an adenoidectomy specimen will be presented. Overall, for all age groups and stages of disease, fewer than 75 cases of Hodgkin's disease with nasopharyngeal involvement have been reported in the world literature. Further, fewer than 15 cases had isolated primary involvement. Although unusual, the occurrence of primary adenoidal Hodgkin's disease in our patient supports the submission of adenoidal tissue for micropathological examination when a relatively large amount of tissue is found at the time of surgery.

Adenoids↗
PubMed1989

Deep venous thrombosis complicating myelomeningocele: report of three cases.

Deep venous thrombosis is a frequent, well-recognized complication of spinal cord injury. Patients with myelomeningocele often have similar weakness of the lower extremities. Following orthopedic surgery, they may also be immobilized at a time when they are hypercoagulable. In addition, as with patients with spinal cord injury, patients with myelomeningocele are prone to urinary tract infection, which may cause local inflammation in the pelvic veins. For the first time, three patients with myelomeningocele complicated by deep venous thrombosis are described. The differential diagnosis (deep venous thrombosis vs osteomyelitis vs fracture) is also discussed in a child with myelomeningocele and a warm, swollen leg, as are the diagnostic methods available. Finally, the issue of antithrombotic prophylaxis in patients with myelomeningocele who are to undergo extensive orthopedic surgery is discussed.

Adolescent↗
PubMed1986

Bone marrow transplantation in lymphomatoid granulomatosis. Report of a case.

Lymphomatoid granulomatosis is an unusual disorder histologically characterized by an angiocentric, angiodestructive mixed cellular infiltrate. The most frequent clinical manifestations are seen in the lungs, the skin, and the central nervous system. Progression to lymphoma may occur, particularly in patients who are anergic. The authors report the case of a boy who had had a splenectomy at 2 years of age for presumed Evan's syndrome, and two episodes of pneumococcal meningitis at 5 and 10 years of age. At 14 years, he had severe respiratory compromise, and a lung biopsy specimen showed lymphomatoid granulomatosis. The liver and bone marrow also were affected. Improvement occurred with multiagent chemotherapy, but he had multiple relapses. A bone marrow transplant was performed using a human leukocyte antigen (HLA) identical mixed lymphocyte culture (MLC) nonreactive brother as the donor. He remains in remission more than 3 years post-transplant. In addition, his abnormal immune function has improved.

Adult↗
PubMed1986

Chromosomal changes in secondary leukemias of childhood and young adulthood.

The increasing success of antineoplastic therapy has resulted in a growing number of long-term survivors. These people are at risk for complications of the therapy itself. Among these induced acute nonlymphoid leukemia (ANLL) has been both common and often lethal. We reviewed 72 recently reported patients under 30 years of age at the time of initial diagnosis who developed a secondary, karyotypically defined leukemia. Fifty-eight patients contracted ANLL a mean of 4 1/2 years from the initial diagnosis. In 25 patients, this was preceded by a preleukemic phase characterized by a hypercellular bone marrow with abnormal precursors, often accompanied by peripheral pancytopenia, that lasted a mean of 6 months. Three additional patients died in this preleukemic phase. In all 61, the most common chromosomal abnormalities were numerical errors. Twenty-four patients had a hypodiploid karyotype, most often in those in whom the primary diagnosis was lymphoma (22 of 43). The most common chromosomes missing in whole or in part were number 7 (18 patients), number 5 (8 patients), number 17 (5 patients), and number 21 (4 patients). The anomalies were frequently multiple and complex. Monosomy 7 figured particularly strongly and may be similar to a karyotypically identical myeloproliferative disorder characterized by micromegakaryocytes, giant platelets, and abnormal granulocyte function arising de novo in children. These findings are similar to those in older patients with ANLL induced by environmental carcinogens or antineoplastic therapy. They are different from the karyotypic changes seen in de novo ANLL in children and young adults, suggesting a different etiology. Also, they reinforce the need to find less leukemogenic treatment programs.

Acute Disease↗
PubMed1986

Mandibular relapse in acute leukemia. Report of a case.

As chemotherapy has become more effective, patterns of relapse have begun to change. We report a patient with T-cell acute lymphoblastic leukemia (ALL) who had an isolated mandibular relapse that was first manifested as dental pain. Eight other recently reported patients with acute leukemia who relapsed in the mandible are reviewed. In four the relapse was isolated to the mandible; all four had ALL. Local control of disease was achieved with radiotherapy, often combined with chemotherapy. However, our patient and two of the other four subsequently relapsed and died of refractory leukemia.

Acute Disease↗
PubMed1986

Acute lymphoblastic leukemia at relapse in a child with acute myeloblastic leukemia.

We describe a child diagnosed as having acute myelogenous leukemia (AML) at 25 months of age who relapsed with acute lymphoblastic leukemia (ALL) 1 year later. The AML was morphologically M2 by the French-American-British classification, periodic acid Schiff (PAS) stain negative and peroxidase positive. The ALL was L1 by this classification, PAS positive, and peroxidase negative. The initial AML was associated with a small segment deletion of the long arm of chromosome 11 [46, XY, de (11) (q23)] not seen in the relapse ALL, which had a normal karyotype. The child was rapidly reinduced with vincristine and prednisone, and remains in remission on maintenance lymphoma type (LSA2-L2) therapy more than 2 years later. These findings suggest the development of a new leukemic clone, rather than a phenotypic modulation of the initial leukemia.

Bone Marrow↗
PubMed1985

Thrombosis as a complication of inflammatory bowel disease in children: a report of four cases.

Thrombosis and thromboembolism have rarely been reported in inflammatory bowel disease (IBD) in children. Four cases of thrombosis, two with apparently serious thromboembolism, are reported in three patients with ulcerative colitis and one with Crohn's disease. Two patients were on steroid treatment at the time of the initial thromboembolic event. Thrombosis may result from a "hypercoagulable state" that occurs in IBD, brought about by a combination of altered clotting factors and the presence of a thrombogenic gastrointestinal mucosa, the risk of which is increased by steroid medications. Children and adolescents with IBD are at risk for serious thromboembolic complications.

Adolescent↗
PubMed1985

Oral lesion in a patient with calcinosis and arthritis: case report and differential diagnosis.

Calcinosis, the process whereby calcium salts are deposited in soft tissues, may be idiopathic, metastatic or dystrophic. Metastatic calcinosis develops in a variety of systemic diseases characterized by either hypercalcemia, hyperphosphatemia, or both. Dystrophic calcinosis refers to calcification of previously damaged or necrotic tissue. It may be found accompanying inflammatory or degenerative conditions and is frequently associated with connective tissue diseases. When pathologic calcification is widespread, an attempt must be made to determine the underlying cause. A case is presented in which there was multifocal calcium deposition in soft tissues, including an intra-oral site. The patient also exhibited severe arthritis, sicca syndrome, focal alopecia and vitiligo. In view of this clinical spectrum, one of the "collagen diseases" (dermatomyositis, lupus erythematosus, rheumatoid arthritis, and scleroderma) was suspected as a predisposing factor for disseminated calcinosis. When diagnostic workup failed to reveal a specific connective tissue disease, it was concluded that "undifferentiated connective tissue disease" was responsible for dystrophic calcinosis.

Aged↗
PubMed1985

The identification of a 'John Doe'.

This paper presents a case report describing the forensic dental investigation that enabled the identification of a decomposed dead body. The body was discovered under such circumstances that there were no known missing persons to account for its occurrence and no clues as to a possible identify. Through teamwork, fragments of nonspecific information were pieced together to point to a possible victim who had disappeared a year earlier and 1,400 miles away. Subtle dental and anthropologic peculiarities were detected and identified on antemortem medical radiographs made 3 years earlier, thus allowing a successful match. The extent of our nation's missing persons and unidentified dead problem is discussed. In a country where almost 40,000 cases of unsolved missing persons are recorded and 2,000 to 10,000 unidentified dead bodies remain anonymous, cooperation among jurisdictions and the use of dental evidence in a computerized form are the likely means of approaching this problem.

Adult↗